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Biomedical subjects

W Kuis

Publications and source records attributed to W Kuis.

At least 37 records · Page 2Linked to original sources
PubMed2001

Effects of sulfasalazine treatment on serum immunoglobulin levels in children with juvenile chronic arthritis.

This article describes the effects of sulfasalazine (SSZ) treatment on serum immunoglobulin (Ig) levels in 6 children with oligoarticular- or polyarticular onset juvenile chronic arthritis (JCA). None of the children who developed dysimmunoglobulinemia during treatment showed clinical symptoms of this adverse event, in particular none developed severe infections. All patients regained normal immunoglobulin levels after discontinuing SSZ treatment. One patient with a partial IgA deficiency at the start of SSZ treatment showed a slow increase in the IgA level during treatment. During follow-up (4-6 years), one patient spontaneously developed a dysimmunoglobulinemia and one patient developed diabetes mellitus. Based on these case reports and review of the literature we advocate monitoring of serum immunoglobulin levels while on SSZ treatment.

Antirheumatic Agents↗
PubMed2001

Clinical and molecular variability in childhood periodic fever with hyperimmunoglobulinaemia D.

OBJECTIVES: The hyperimmunoglobulinaemia D and periodic fever syndrome (HIDS) was found recently to be caused by a deficiency of mevalonate kinase (MK). The aim of this study was to examine whether a relationship exists between the clinical expression of HIDS and the extent of MK deficiency. METHODS: The medical records of children diagnosed with HIDS were reviewed for clinical features and serum immunoglobulin values. The mevalonic acid excretion in urine and MK enzyme activity in patients' cells were measured and the cDNA of the MVK gene was sequenced. RESULTS: Fifteen patients with recurrent fever and raised serum immunoglobulin (Ig) D were included. Their clinical features varied. Eleven patients had a deficiency of MK, caused by mutations in the MVK gene. One mutation (V377I) was common to all 11 patients. Nine patients were compound heterozygotes for V377I and various other MVK mutations. There was no apparent relationship between the observed mutations and the clinical features. Surprisingly, four boys had normal MK activity and no MVK mutations. CONCLUSIONS: Most HIDS patients have mutations in the MVK gene. The clinical variability observed cannot be explained by genotypic differences. Periodic fever and elevated IgD can result from other, still unknown, causes. Hence, testing for MK deficiency is necessary in patients with unexplained periodic fever.

Child↗
PubMed2001

Psychological adjustment of adolescent girls with chronic fatigue syndrome.

OBJECTIVE: To examine psychosocial problems and adaptation of adolescent girls with chronic fatigue syndrome (CFS). METHODOLOGY: Thirty-six adolescent girls with CFS (mean age: 15.2 years; mean syndrome duration: 19.7 months) who fulfilled the criteria of the Centers for Disease Control and Prevention were examined by interviews regarding premorbid problems and by questionnaires regarding psychosocial functioning and distress, psychological attitudes, and coping resources. Data were compared with normative data. RESULTS: Of the adolescents, 86.1% reported 1 or more premorbid problems (58.3% physical, 38.9% psychological, and 52.8% familial). Normal adjustment was reported for psychosocial self-esteem, social abilities, and attentional abilities. High adjustment to adult social standards of behavior was found, but low perceived competence in specific adolescent domains, such as athletic ability, romance, and participation in recreational activities. The girls reported predominantly internalizing problems. Normal achievement motivation, no debilitating fear of failure, and high internal locus of control were observed. Palliative reaction patterns and optimism were predominantly used as coping strategies. CONCLUSIONS: The large number of premorbid problems suggests a possible contributing factor to the onset of the syndrome, although there were no reference data of healthy adolescents. In distinct domains of psychosocial adjustment, the adolescent girls with CFS showed strengths such as adequate self-esteem and scholastic and social abilities, and weaknesses such as low competence in adolescent-specific tasks and internalizing distress, which may partly be explained by syndrome-specific somatic complaints. The use of optimistic and palliative reaction patterns as coping strategies in this patient group indicates that the patients with CFS seem to retain an active and positive outlook on life, which may result in a rather adequate psychological adaptation to the syndrome, but also in maintenance of the syndrome by exceeding the physical limits brought about by the CFS. Our results on adjustment and coping strategies may be helpful to implement (individual) rehabilitation programs.

Adaptation, Psychological↗
PubMed2000

Stress induces increases in IL-6 production by leucocytes of patients with the chronic inflammatory disease juvenile rheumatoid arthritis: a putative role for alpha(1)-adrenergic receptors.

Juvenile rheumatoid arthritis (JRA) is characterized by chronic inflammation of the joints. In the present study we demonstrate that exposure of JRA patients to a noradrenergic stressor (cold pressor test) results in enhanced LPS-induced IL-6 production by peripheral blood cells of these patients. Healthy, age-matched controls had the same rise in norepinephrine, but do not respond with changes in IL-6 production after exposure to the cold pressor test. Moreover, PBMC of patients with JRA express mRNA encoding alpha(1)-adrenergic receptors (AR), predominantly of the alpha(1d)-AR subtype. In contrast, we could not detect mRNA encoding for alpha(1)-AR in PBMC of healthy controls. The results of this study suggest that expression of alpha(1)-AR mRNA in PBMC during chronic inflammation is associated with altered responses of the immune system to stress.

Acute Disease↗
PubMed2000

[Identification of the gene for hyper-IgD syndrome: a model of modern genetics].

Hyperimmunoglobulinaemia D and periodic fever syndrome (HIDS) is a rare autosomal recessive disorder. Patients suffer from recurrent attacks (3-6 days) with fever, abdominal distress, lymphadenopathy, skin lesions and arthralgias. Patients display a constantly elevated serum IgD which serves as a biological marker of the disease. Recently, the gene for HIDS was discovered by two independent groups using positional and functional cloning methods. One group used linkage analysis (positional cloning) and was able to locate the gene for HIDS on the long arm of chromosome 12 (12q24). Mevalonate kinase was an interesting candidate gene because patients with a near complete absence of this enzyme (mevalonic aciduria) do exhibit attacks of fever. Indeed subsequent data showed that there was a decreased enzyme activity due to missense mutations in the mevalonate kinase gene. The other group detected slightly elevated urinary excretion of mevalonic acid during attacks in a HIDS patient (functional cloning). The enzyme activity of mevalonate kinase was lower in cultured cells and sequence analysis identified several missense mutations in cDNA encoding for mevalonate kinase. Mevalonate kinase is a key enzyme in the cholesterol synthesis pathway and it is rather surprising that a defect in the cholesterol metabolism can cause a periodic inflammatory disease such as HIDS.

Biomarkers↗
PubMed2000

Myeloid-related proteins 8 and 14 are specifically secreted during interaction of phagocytes and activated endothelium and are useful markers for monitoring disease activity in pauciarticular-onset juvenile rheumatoid arthritis.

OBJECTIVE: To analyze which physiologic stimuli induce secretion of myeloid-related protein 8 (MRP8) and MRP14, two S100 proteins expressed in neutrophils and monocytes, and to determine whether serum concentrations of these proteins are reliable parameters for monitoring inflammatory activity in pauciarticular juvenile rheumatoid arthritis (JRA). METHODS: Secretion of MRP8 and MRP14 was analyzed using a coculture system of endothelial cells and monocytes. Concentrations of MRP8/MRP14 in the serum and synovial fluid of JRA patients or culture medium were determined by enzyme-linked immunosorbent assay. The expression of MRP8 and MRP14 by leukocytes in synovial tissue or fluid was investigated using immunohistochemistry. RESULTS: MRP8 and MRP14 were specifically released during interaction of activated monocytes with tumor necrosis factor-stimulated endothelial cells. Secretion was mediated via an increase in intracellular calcium levels in monocytes. In contrast, contact with resting endothelium inhibited protein kinase C-induced secretion of the proteins by monocytes. In JRA patients, MRP8 and MRP14 were strongly expressed in infiltrating neutrophils and monocytes within the inflamed joints and could be found in significantly higher concentrations in synovial fluid (mean 42,800 ng/ml) compared with serum (2,060 ng/ml). Concentrations of MRP8/MRP14 in serum correlated well with those in synovial fluid (r = 0.78) and showed a strong correlation with disease activity (r = 0.62). After intraarticular triamcinolone therapy, the serum concentrations of MRP8/MRP14 decreased significantly in therapy responders, whereas no differences were found in patients who showed no clinical benefit. CONCLUSION: MRP8 and MRP14 are specifically released during the interaction of monocytes with inflammatory activated endothelium, probably at sites of local inflammation. Their serum concentrations represent a useful marker for monitoring local inflammation in JRA.

Adolescent↗
PubMed2000

Displacement response of juvenile arthritic wrists during grasp.

OBJECTIVE: To analyze the displacement response of juvenile arthritic wrists during grasp in order to diagnose early ligamental laxity and facilitate early splinting. METHODS: X-rays of the wrists, made under standardized conditions, of 30 children with juvenile chronic arthritis (mean age 10.4 years, range 4.5-16.9) were analyzed after being digitalized. Osseous landmarks were identified, and coordinates were calculated from measured angles and lengths with an accuracy of 0.01'. Lunate and carpal-ulnar distance were obtained according to Youm, and ulnar variance according to Häfner. RESULTS: Overall, an increase in ulnar-lunate displacement and carpal narrowing and a decrease in ulnar variance were found. However, not all wrists responded to the same extent. Radial displacement of the lunate, though slight, was found in 2 wrists and the amount of ulnar displacement varied substantially (3.1% to 22.5%). The variance in amount of displacement could suggest that juvenile wrists do not respond to increased compressive forces to the same extent. CONCLUSION: The changes found are similar to those found in the healthy wrist. Furthermore, our findings suggest that the juvenile wrist acts in accordance with the generally accepted explanation for the development of malalignment in adult wrists. It seems that laxity of ligaments can be diagnosed early by the force grip maneuver during x-ray. It would have a significant impact on the moment of orthotic intervention as well as the design of the orthotic device. Further study along this line seems justified.

Adolescent↗
PubMed2000

Autologous hemopoietic stem-cell transplantation for children with refractory autoimmune disease.

Autologous stem cell transplantation (ASCT) has been proposed as a possible treatment for severe autoimmune diseases such as rheumatoid arthritis (RA), multiple sclerosis (MS), systemic sclerosis, and systemic lupus erythematosus (SLE). To date, more than 250 patients with various autoimmune disorders have undergone an ASCT since 1996. Among them, there is a very limited number of children. This review summarizes the experience with ASCT for pediatric rheumatic diseases. Most reported cases concern juvenile idiopathic arthritis (JIA). Experience with ASCT for childhood SLE, Scleroderma, or Dermatomyositis is very limited. To date, 12 children with severe systemic or polyarticular JIA, all with progressive disease activity despite the use of corticosteroids, MTX, CsA, or Cyclophosphamide were treated in our center with ASCT. Rheumatologic follow-up at 3-month intervals up to 36 months showed a marked decrease in arthritis severity as expressed by the core-set criteria for juvenile chronic arthritis (JCA) activity. However, these children remain at risk for severe viral infections due to the prolonged lymfopenia. ASCT in this severely ill patient group induces a very significant and drug-free remission of the disease, but carries a significantly risk of developing fatal MAS.

Adolescent↗
PubMed2000

The influence of the hormonal system on pediatric rheumatic diseases.

The role of NEI interactions in children with chronic inflammatory rheumatic disorders has not been systematically studied to the same extent as in adults. The data that are currently available suggest that NEI mechanisms are involved in the pathophysiology of some of the diseases. These include JRA, JSLE, and JAS. Prolactin has been most extensively investigated, showing interesting parallels with findings in adult rheumatologic diseases. Limited data on cortisol suggest a deficiency of production in JRA, a situation similar to that in adult RA. These findings suggest that there is a proinflammatory hormonal bias in children with JRA and JSLE. The data in children with chronic autoimmune inflammatory disorders seem to be identical to those seen in adults with RA and SLE, but a clear delineation of the role of the neuroendocrine-immune system in disease pathophysiology is still required. The neuroendocrine aspects of pediatric rheumatologic disease observed to date suggest a number of avenues for further research in the field of neuroendocrine immunology, which may open up novel therapeutic options.

Adolescent↗
PubMed2000

Favourable outcome in 135 children with juvenile systemic sclerosis: results of a multi-national survey.

OBJECTIVE: To increase the current knowledge of the outcome of juvenile systemic sclerosis (jSSc), which is currently limited. METHODS: In order to investigate the patient outcome and prognostic factors, starting October 1994, we distributed questionnaires to 324 paediatric rheumatology centres. RESULTS: Until 15 May 1998 responses from 46 centres were received, 34 of which returned completed questionnaires on a total of 135 patients. One hundred and twenty-two of the 135 patients were Caucasian, 100 were female. The mean age at disease onset was 8.8 yr (S.D. +/- 3.3 yr). The mean disease duration at the last follow-up was 5 yr(S.D. +/- 3.3 yr). At the last follow-up the disease was still active and required medication in 82 patients, 36 had inactive disease on medication, and 16 were in remission. Ninety per cent of the living patients were fully active in daily life at the last follow-up. Eight of the 135 patients had died. These patients had a median age at onset of the disease of 10.5 yr (range 6.7-15.8 yr). The median disease duration until death was 2 yr (range 1-8 yr). The causes of death were heart failure (five), renal failure (one), sepsis (one) and in one case the cause was not defined. The 1 yr survival rate was 99%, the 2 yr was 97% and the 4 yr was 95%. CONCLUSIONS: At a mean follow-up of 5 yr, the current results show a favourable outcome in most patients with childhood onset jSSc and a significantly better survival than in the adult SSc patients.

Adult↗
PubMed2000

Psychological, behavioural, and social adjustment in children and adolescents with juvenile chronic arthritis.

OBJECTIVE: To assess the psychological, behavioural and social adjustment of children (7-11 years) and adolescents (12-16 years) with juvenile chronic arthritis (JCA). Higher rates of maladjustment were expected to be found in these patients. METHODS: Self report questionnaires were used within the context of personal interviews. Family functioning and social support were studied as well. Forty seven patients with JCA, 52 healthy peers and their respective parents participated in the study. RESULTS: Self esteem, perceived competence and body image in patients with JCA were as positive as they were in healthy participants. There were no differences between ill and healthy youngsters with respect to the incidence of psychopathology. Patients with JCA, in general, perceived themselves as socially competent, but they seemed to have somewhat less opportunity or energy to participate in social activities. Children with JCA showed a high level of aspiration to cope with social expectations. This aspiration seemed to be even stronger in case the disease caused more strains, for example, in periods of inflammation and in the systemic onset type. The high level of social adjustment in children with JCA seemed to be supported by highly cohesive family structures. Generally, adolescents with JCA experienced much social support. CONCLUSIONS: In contrast with our expectation, children and adolescents with JCA seemeed to cope quite well with the psychological and social consequences of their long term condition. For future studies, it is hypothesised that the high levels of adaptation might imply an enduring psychological strain, which is reflected in an altered function of the autonomic nervous system.

Adolescent↗
PubMed2000

High nocturnal melatonin in adolescents with chronic fatigue syndrome.

Decreased quality of sleep is frequently reported by chronic fatigue syndrome (CFS) patients. The pineal hormone melatonin is involved in regulation of sleep. We analyzed the nocturnal rise in melatonin in 13 adolescent CFS patients and 15 healthy age-matched controls. Saliva samples were collected at hourly intervals between 1700 and 0200 h. Nocturnal saliva melatonin levels were significantly higher in CFS patients, compared with controls, at midnight, 0100 h, and 0200 h (P < 0.001). No differences were observed in timing of melatonin increase in saliva between patients and controls. Time of sleep onset and duration of sleep did not differ significantly between patients and controls. However, all CFS patients and only one of the controls in our study group reported unrefreshing sleep. Our data demonstrate that sleep problems in adolescents with CFS are associated with increased melatonin levels during the first part of the night. Based on these data, we suggest that there is no indication for melatonin supplementation in adolescents with CFS.

Adolescent↗
PubMed2000

Disturbed neuroendocrine-immune interactions in chronic fatigue syndrome.

The present study was designed to investigate the interaction between neuroendocrine mediators and the immune system in chronic fatigue syndrome (CFS). We examined the sensitivity of the immune system to the glucocorticoid agonist dexamethasone and the beta2-adrenergic agonist terbutaline in 15 adolescent girls with CFS and 14 age- and sex-matched controls. Dexamethasone inhibits T-cell proliferation in healthy controls and in CFS patients. However, the maximal effect of dexamethasone on T-cell proliferation is significantly reduced in CFS patients as compared with controls. The beta2-adrenergic receptor agonist terbutaline inhibits tumor necrosis factor-alpha production and enhances interleukin-10 production by monocytes. Our data demonstrate that the capacity of a beta2-adrenergic agonist to regulate the production of these two cytokines is also reduced in CFS patients. We did not observe differences in baseline or CRH-induced cortisol and ACTH between CFS patients and controls. Baseline noradrenaline was similar in CFS and controls, whereas baseline adrenaline levels were significantly higher in CFS patients. We conclude that CFS is accompanied by a relative resistance of the immune system to regulation by the neuroendocrine system. Based on these data, we suggest CFS should be viewed as a disease of deficient neuroendocrine-immune communication.

Adolescent↗
PubMed1999

Autologous haemopoietic stem-cell transplantation in four patients with refractory juvenile chronic arthritis.

BACKGROUND: Autologous haemopoietic stem-cell transplantation (AHSCT) had been described as a possible treatment for severe autoimmune disease refractory to conventional treatment. We report the first four children with severe forms of juvenile chronic arthritis (JCA) treated with AHSCT. METHODS: We studied three children with systemic JCA and one child with polyarticular JCA. Unprimed bone marrow was taken 1 month before AHSCT. T-cell depletion of the graft was done with CD2 and CD3 antibodies. We used a preparative regimen of antithymocyte globulin (20 mg/kg), cyclophosphamide (200 mg/kg) and low-dose total body irradiation (4 Gy). Methotrexate and cyclosporin were stopped before AHCST, prednisone was tapered after 2 months. FINDINGS: Our patients showed a drug-free follow-up of 6-18 months with a marked decrease in joint swelling, pain, and morning stiffness. Erythrocyte sedimentation rate, C-reactive protein, and haemoglobin returned to almost normal values within 6 weeks. Despite T-cell depletion there was a rapid immune reconstitution in three out of four children. Two patients developed a limited varicella zoster virus eruption, which was treated by aciclovir. INTERPRETATION: AHSCT for severe JCA was well tolerated and induced a remission of disease in four children with JCA that was resistant to conventional treatment. Prolonged prednisone-free growth catch-up and general well-being is a major therapeutic gain in such children. The actual follow-up is too short, however, for us to conclude that these children are completely cured of their disease.

Arthritis, Juvenile↗